Basilar invagination presenting as recurrent posterior circulation stroke with locked-in syndrome in a child: a case report

Abstract

Basilar invagination is a type of craniovertebral junction anomaly which occurs when the tip of odontoid process migrates upward into the intracranial space through foramen magnum and most commonly present as progressive neurological deficit implicating the high cervical cord, lower brainstem and cranial nerves. It can rarely present as vertebro-basilar territory insufficiency and occlusion, leading to stroke. Treatment is typically surgical and continues to centre on varying combinations of anterior or posterior decompression with or without traction or fusion. We present a 13-year-old boy with basilar invagination diagnosed on MRI with angiogram, which was missed on initial presentation, leading to recurrent posterior circulation stroke progressed to Locked-in syndrome. This case report emphasis the need of screening for craniovertebral junction anomalies in young patients presenting with posterior circulation stroke.

Two Different Cases of Stroke in Young

“…Basilar invagination is known for dynamic flow reduction and vertebrobasilar insufficiency during neck movements (11).Isolated atrial septal aneurysym has causal association with stroke in young (12). Combination of these conditions, as in our case require guidelines for further management.…”

“…In our case scenario, marfan syndrome associated with platybasia and isolated atrial septal aneurysym with recurrent posterior circulation stroke will think in favour of either vertebrobasilar compromise from dens impinging on medulla oblongata or moving thrombi from isolated ASA or due to arrythmia.As per Kumar et al basilar invagination will increase the chances of vertebrobasilar stroke by direct compression on neurovascular structures (11).There are lot of ultasonagraphic studies on dyanamic flow reduction during neck movements for documenting VBI. In our case MR neck and intracranial angiogram showed normal flow during the study.…”